title: Endothelin receptor antagonists.
abstract: Three pathways have been identified in the pathogenesis of pulmonary arterial hypertension (PAH): the endothelin (ET), nitric oxide (NO) and prostacyclin pathways. These pathways represent the targets of approved PAH therapies and their discovery has facilitated significant progress in the understanding and treatment of PAH. The ET system is well established as a key player in the pathophysiology of PAH, with deleterious effects mediated by both the ETA and ETB receptors. Endothelin receptor antagonists (ERAs) are an important part of PAH therapy, with two ERAs currently approved for the treatment of PAH and a novel ERA that has recently been investigated in a Phase III clinical trial. This chapter describes the role of ET in the pathogenesis of PAH, reviews experimental data and examines the clinical status of ERAs in PAH treatment.
authors.affiliation: Actelion Pharmaceuticals Ltd, Gewerbestrasse 16, 4123, Allschwil, Switzerland, martine.clozel@actelion.com.
authors.forename: Martine
authors.initials: M
authors.lastname: Clozel
authors.forename: Alessandro
authors.initials: A
authors.lastname: Maresta
authors.forename: Marc
authors.initials: M
authors.lastname: Humbert
mesh_terms: D000818:Animals; D006332:Cardiomegaly / Q000517:prevention & control; D004347:Drug Interactions; D065128:Endothelin Receptor Antagonists; D065627:Familial Primary Pulmonary Hypertension; D005355:Fibrosis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy* / Q000209:etiology; D009206:Myocardium / Q000473:pathology; D017466:Receptors, Endothelin / Q000502:physiology
chemical_list: D065128:Endothelin Receptor Antagonists; D017466:Receptors, Endothelin